Primary Thoracic Chondrosarcoma with Intra‑Abdominal Extension in a Renal Transplant Recipient: a Case Report

MOLECULAR AND CLINICAL ONCOLOGY 13: 63-66, 2020

Primary thoracic chondrosarcoma with intra‑abdominal extension in a renal transplant recipient: A case report

DIMITRIOS GIANNIS1, DIMITRIOS MORIS2,3, BRIAN ISHUM SHAW2 and SPYRIDON VERNADAKIS3

1Faculty of Medicine, School of Health Sciences, University of Thessaly, 41110 Larissa, Greece; 2Duke Surgery, Duke University Medical Center, Durham, NC 27710, USA; 3Transplantation Unit, Laiko General Hospital, 11527 Athens, Greece

Received September 11, 2019; Accepted February 18, 2020

DOI: 10.3892/mco.2020.2034

Abstract. Primary thoracic bone tumors are relatively rare. such as enchondromas, and are usually located in bones that The most common type is chondrosarcoma, accounting for up undergo endochondral ossification (1,7,8). Early recognition, to 48% of all cases. Patients with primary thoracic bone tumors preoperative evaluation and adequate surgical intervention commonly present with atypical thoracic pain or a solitary with wide margins are crucial to prevent invasive local disease palpable chest mass, which gradually develops over months and metastasis (3,4,8). Awareness of the characteristics and to years. The bones most often affected are the ribs, scapula, behavior of these tumors are necessary in order to avoid costochondral junctions and the sternum. The present study misdiagnosis and inadequate or unnecessary therapeutic presents a case of a 79 year old previous transplant recipient interventions (4). with a large intra‑abdominally expanding chondrosarcoma De novo malignancies have become one of the leading originating from the left lower thoracic cage and associated causes of late mortality after renal transplantation, with their vague abdomdinal symptoms. Early recognition and aware- incidence being 2‑15-fold higher than in general population (9). ness of atypical presentations of this disease are important in We report a case of large thoracic chondrosarcoma in a kidney to appropriately guide diagnostic evaluation and therapeutic transplant recipient with intra‑abdominal invasion combined procedures. with vague gastrointestinal‑related symptoms.

Introduction Case report

Primary tumors of the chest wall, arising from soft tissue, A 79‑year‑old woman was referred to our clinic with 3 month bone or cartilage are uncommon. At most, the reported history of mild epigastric pain and intermittent nausea, incidence is only 500 new cases per year in the United without vomiting episodes. The patient underwent kidney States (1). Primary bone tumors of the thoracic cage are rela- transplantation from deceased donor for end stage renal tively rare, representing up to 4‑8% of all non‑secondary, disease due to diabetic nephropathy 20 years ago, complicated non‑metastatic bone neoplasias (2,3). Most chest wall by chronic rejection and return to dialysis the last 5 years. Her bony tumors demonstrate malignant behavior and the most immunosuppression regimen included steroids, cyclosporine, common primary malignancy is chondrosarcoma (1‑4). and mycophenolate mofetil. Given the clinical presentation Waller and Newman (2), using data from the Leeds bone and examination findings, a chest and abdomen computerized tumor registry, reported that chondrosarcoma constituted tomography (CT) scan was performed at an outside facility in around 24% of all bone tumors of chest wall, while other order to identify the origin of the palpable mass. Per report, estimates place its incidence at 15‑48.1% of the thoracic CT revealed a large space‑occupying, non‑homogeneous wall malignancies (4‑6). solid mass accompanied by intralesional calcifications and Chondrosarcomas may arise as de novo lesions or in the measuring approximately 17x15x12 cm (data not shown). In an context of preexisting benign tumors of cartilage origin, effort to better characterize the mass and delineate its margins, the patient underwent a magnetic resonance imaging (MRI) scan one week later. It demonstrated a mass arising from the 9th left rib with infiltration of the adjacent subcutaneous Correspondence to: Dr Dimitrios Moris, Transplantation Unit, fat tissue. Additionally, the mass exhibited intra‑abdominal Laiko General Hospital, Agiou Thoma 17, 11527 Athens, Greece subdiaphragmatic extension with compressive effects on E‑mail: [email protected] spleen, greater curvature of the stomach, body and tail of pancreas, left colic flexure, left kidney and renal vein. The Key words: chondrosarcoma, chest wall tumor, rib tumor, sarcoma lesion was well‑circumscribed and demonstrated imaging characteristics of neovascularized osteochondral tissue. Preoperative MRI of chest and abdomen did not reveal any suspicious intraparenchymal organ lesion or pathologically 64 GIANNIS et al: PRIMARY THORACIC CHONDROSARCOMA WITH INTRA-ABDOMINAL EXTENSION

Figure 2. Postoperative 12 month coronal and transverse plane MRI. (A) Coronal plane MRI revealing no signs of local tumor recurrence. (B) Transverse plane MRI revealing absence of tumor recurrence and no Figure 1. Preoperative coronal and transverse plane MRI. (A) Coronal plane signs of intraparenchymal organ lesions. MRI demonstrating the presence of a well‑circumscribed, space‑occupying, non‑homogeneous solid lower thoracic mass with intra‑abdominal extension. (B) Transverse plane MRI presenting the intra‑abdominal extension of the mass and the absence of intraparenchymal organ lesions. left sided chest tube and left subdiaphragmatic drain were also placed. Subsequently, the abdominal wall incision was closed in anatomic layers with simultaneous repair of 2 supraumbilical hernias caused by previous laparotomy. The patient, was trans- enlarged intra‑abdominal, retroperitoneal or pelvic lymph ferred to the intensive care unit intubated and hemodynamically nodes (Fig. 1). stable for monitoring and further postoperative management. Considering the clinical presentation and imaging The patient was gradually weaned off the ventilator during findings, the decision was made to proceed with surgical the 3rd postoperative day and was subsequently transferred to exploration and resection of the mass with curative intent. the surgical ward. Her diet was advanced gradually and the Exploratory laparotomy was initiated with a left Kocher inci- abdominal drain was removed on post‑operative day 8, and the sion extended to the xiphoid process and right subcostal area. chest tube was removed 2 days later. Follow‑up radiographic Intraoperatively, a botryoid, grayish lesion of cartilaginous imaging revealed minimal left pleural effusion without any consistency was identified. Its smooth, membranous external signs of pneumothorax. Histopathologic examination reported surface was carefully detached from the surrounding tissues the presence of Grade II chondrosarcoma with malignant with blunt dissection. Despite being in close proximity to the invasion of the 9 and 10th ribs. The patient was discharged mass capsule, the adjacent organs, such as the spleen, greater on the post‑operative day 14 and referred to medical oncology curvature of the stomach, pancreas, colon and left renal vein for further evaluation and management. The patient remained were not invaded. A portion of the diaphragm measuring disease‑free at the 12 month follow‑up, with no evidence of 12x12 cm was invaded and removed via the abdominal inci- disease on MRI (Fig. 2). sion. It corresponded to the left costophrenic recess as well as The present study was approved by the Ethics Committee 9 and 10th lateral rib shaft parts. of Laiko General Hospital (Athens, Greece). Data analysis was The diaphragmatic defect was repaired with mesh. The performed in accordance with the Declaration of Helsinki. visceral antiadhesive surface was placed adjacent to the left Patients who participated in this research had complete clinical lower lung lobe and the parietal surface put in contact with the data. The signed informed consents were obtained from the spleen in order to avoid adhesion to the small bowel serosa. A patients or the guardians. MOLECULAR AND CLINICAL ONCOLOGY 13: 63-66, 2020 65

Discussion tomography (PET) is occasionally used to identify metastatic disease and has also been suggested as a diagnostic modality Soft tissue sarcomas are rare tumors of mesenchymal to differentiate between benign chondral tissue tumors and origin (10). In transplant patients, with the exception of Kaposi chondrosarcoma (1,20). sarcoma‑which is relatively common with a 400‑500-fold As chondrosarcomas are resistant to radiation and most increased incidence‑soft tissue sarcomas are rarely encoun- chemotherapeutic agents, resection considered first line treat- tered, thus the diagnostic and treatment algorithms follow ment (7,14,16). Surgical intervention involves tumor excision those of general population (11‑13). with 3‑4 cm margins in order to prevent local disease recur- Chondrosarcoma is the most common primary rence (7). However, it is not always feasible to achieve these malignancy of the thoracic cage and is usually located in margins due to proximity to vital neurovascular and visceral bony tissue developing through the endochondral ossification structures (7,16). Local tumor recurrence, metastasis, and process (1,3). These malignant neoplasms may arise as lesions survival are affected by tumor grade, size, diameter, location in previously healthy bone tissues or develop in preexisting and positive margins (7,14,21). Grade I chondrosarcomas benign chondral tissue tumors, such as enchondromas (1). have better prognosis compared to Grade II and III tumors, In addition, chondrosarcomas may develop centrally, in the with 10‑years survival of 83, 64 and 29% respectively (17). periphery or on the periosteal surface of the bone where they McAfee et al reported a higher local recurrence rate in patients most commonly affect the ribs, the costochondral junction and treated with excision with negative margins alone compared less frequently the sternum (1,4,14). to a 3‑4 cm margin (50 vs. 17%) (21). In addition, 10‑year Chondrosarcomas are reported to account for 15‑48.1% of survival dropped from 96% in wide margin group to 65% in the thoracic wall malignancies (2,4‑6). Burt et al reported that local excision (21). The prognosis is more favorable in patients chondrosarcomas of the chest wall correspond to 14.7% of all with sternal tumors and tumors measuring <6 cm, whereas chondrosarcomas and most of them originated in ribs (43%) survival is <2 years in patients with Grade III tumors (21). and scapula (36%) (15). These tumors predominately affect We present a rare case of chest wall chondrosarcoma in a men with a median age of 50 (1). However, tumors have been previous kidney transplant recipient, with vague symptoms reported in patients as young as 11 and as old as 76 (7,16). Risk due to intra‑abdominal expansion and invasion. Chest wall factors associated with the development of these neoplasms chondrosarcomas are rare tumors, especially in transplant popu- include prior trauma and radiation exposure (often for the lations, which are resistant to chemotherapy and radiotherapy treatment of breast cancer or lymphoma), though causation is and mainly treated with wide local excision. Patients most not well established (1). commonly present with insidious thoracic pain or solitary chest Chondrosarcoma grading is an important predictive factor mass gradually developing over months or years. Awareness of of local recurrence, distant metastasis and patient survival (17). the clinical presentation, appropriate treatment and occasion- Conventional primary chondrosarcomas are histologically ally atypical progression of these tumors are important to avoid graded as Grade I (low cellularity, abundant matrix, rarely unnecessary diagnostic tests and therapeutic interventions. metastasize), Grade II (mitoses present, less chondroid matrix) and Grade III (undifferentiated and highly cellular tumors, Acknowledgements with muco‑myxoid matrix and mitoses) (17). Most patients with chest wall chondrosarcoma present Not applicable. with a gradually increasing, palpable, and usually painful mass arising from an anterior chest wall structure such as Funding the costochondral junction or the sternal surface (1). Tumors arising from the anterior chest wall are usually bidirectional No funding was received. (growing outward and inward) and those arising from the posterior thoracic wall tend to grow inwardly only (16,18). Availability of data and materials Radiographic characterization of these tumors should be the initial step to further guide the appropriate treatment and The datasets used and/or analyzed during the present study are management (19). Patients presenting with chest wall masses available from the corresponding author on reasonable request. should initially be investigated with a posterior‑anterior and lateral thoracic X‑rays (1). After this, CT of the chest, Authors' contributions including neck area and upper abdomen, is considered as the gold‑standard imaging test for accurate tumor localization and DM and SV conceived and designed the present study. DG, preoperative planning (1). On CT, chondrosarcomas charac- DM and SV collected the data. DG and BIS analyzed and teristically appear as well‑circumscribed, lobular masses with interpreted the data. DG, DM and BIS wrote the manuscript. intralesional focal, nodular or peripheral calcifications accom- DM, BIS and SV critically revised the manuscript. All authors panied by adjacent bone or soft tissues invasion (19). MRI does contributed equally to this work. All authors read and approved not offer any diagnostic benefit compared to CT and it is not the final manuscript. routinely performed in the investigation of suspected thoracic chondrosarcoma (1). In paravertebral, mediastinal or thoracic Ethics approval and consent to participate outlet tumors, where neurovascular invasion is a concern, MRI offers additional information as it offers greater ability The present study was approved by the Ethics Committee of to characterizing soft‑tissue structures (1). Positron emission Laiko General Hospital (Athens, Greece). Data analysis was 66 GIANNIS et al: PRIMARY THORACIC CHONDROSARCOMA WITH INTRA-ABDOMINAL EXTENSION performed in accordance with the Declaration of Helsinki. 10. Adam MA, Moris D, Behrens S, Nussbaum DP, Jawitz O, Turner M, Lidsky M and Blazer D III: Hospital volume threshold Patients who participated in this research had complete clinical for the treatment of retroperitoneal sarcoma. Anticancer Res 39: data. The signed informed consents were obtained from the 2007‑2014, 2019. patients or the guardians. 11. Zavos G, Moris D, Vernadakis S, Bokos J, Lionaki S, Mamarelis G, Panagiotellis K, Zavvos V and Boletis I: Incidence and management of Kaposi sarcoma in renal transplant recipients: The Greek Patient consent for publication experience. Transplant Proc 46: 3199‑3202, 2014. 12. Vernadakis S, Moris D, Delimpalta C, Bokos J and Zavos G: Ovarian carcinosarcoma in a renal transplant recipient. A unique Written informed consent was obtained from the patient for case of a rare tumor. Hippokratia 18: 364‑365, 2014. the publication of all accompanying images and data. 13. Vailas MG, Vernadakis S, Moris D and Zavos G: Surgical dead end in a renal transplant recipient associated with a rare throm- bohemorrhagic syndrome. Transplant Proc 47: 2537‑2540, 2015. Competing interests 14. Le HV, Wadhwa R, Theodore P and Mummaneni P: Excision of thoracic chondrosarcoma: Case report and review of literature. The authors declare that they have no competing interests. Cureus 8: e708, 2016. 15. 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